You never know when or if something might spring up with TSC. We came so close after surgery to having some normalcy, but it wasn’t to be just yet. About a month after surgery, on Wednesday August 22, Connor had a follow up EEG. We hadn’t seen any seizures since surgery, and now we would see how he was doing as far as the subclinical seizures that can’t be seen to the naked eye. The neurologist would read the EEG and call us with the results within a week. But on Friday morning, August 24, the moment that had lost me countless hours of sleep and stress happened. We saw his first infantile spasms. I don’t even know how much desperate begging and praying I had done that he wouldn’t develop this complication. Infantile spasms are extremely rare in the general population, but kids with TSC are at a higher risk of developing them. I had watched countless YouTube videos other parents had loaded to help others with identification, so that I would be sure if the moment came. But why so much more concern over this seizure type than what he had before? IS can cause brain damage, prevent children from reaching milestones, and even cause regression. Some people had reported their babies had regressed to a newborn state after developing these, losing any milestones they had achieved.
I had obsessed and obsessed over how things would go when these started. How long would it take to get treatment rolling? How many would he have before treatment started to work? What damage would be done between the moment we first saw them and the moment treatment became effective? One thing I didn’t worry about was whether the drug I knew he would get if this happened, Sabril (vigabatrin). would work. I’d heard amazing things about its effectiveness. I’d even obsessed over whether they would start on a Friday after the neurologist’s office closed further complicating getting treatment started. If these had come out of nowhere, meaning we knew nothing of his TSC, these would warrant going to the ER to get treatment started. But since we already had a plan in place and a neurology team, we were told just to call the office and we’d get the ball rolling. So naturally, they started on a Friday. And the office closes early on Fridays. Did I mention I had a full blown anxiety attack on top of everything else? Because people were right. They said you’d know the spasms when you saw them. And I did.
Click to see spasms before starting Sabril:
It actually worked out well that the office was closed. Instead of going through medical assistants, I was able to directly page the doctor on call. I described what I saw and I could tell my description concerned him. They hadn’t yet reviewed his EEG, so he said he would look at that and call us back shortly. Confirmed. His EEG showed hipsarrhythmia, the scrambled pattern of brainwaves that often (but not always) accompany IS. They called in a prescription for Klonipin (clonazepam) for the weekend to help calm them, and we had the first appointment Monday morning to get him on Sabril. On the bright side, the surgery had been a success. No complex partial seizure activity. We should have been able to celebrate, but I try not to be bitter. Try.
It was a long weekend in which we saw 3-4 clusters a day (his worst day having 8). A cluster is a series of spasms. Monday morning we signed our life away on paperwork due to the fact that a possible side effect of Sabril is damage to or loss of peripheral vision. But as Wendi (the fellow TSC mom who has been there to help us and answer our questions through all this) said, “What good is 20/20 vision if their brain is mush?” I haven’t come across any stories yet about children experiencing this so hopefully he won’t either. Because of the risk, he has to undergo eye exams every three months as a precaution, including an ERG (electroretinography) at Scottish Rite. He has to be sedated and electrodes are used to check his vision. Can nothing be simple? You can’t even get Sabril at a regular pharmacy. It has to be Fedexed to your house.
After about a week and a half we seemed to be in the clear. We went three days with no spasms. Then the eye rolling started. It is now mid October and we are still trying to get a handle on the eye rolling. Presumably, they are milder spasms that are breaking through on the Sabril, but he’s doing well and continues to progress. While the full spasms were going on, he was quieter and it was harder to get a laugh, but he’s back to his giggly self. He underwent an 8-hour EEG to try and determine what was going on. Naturally, he didn’t have a single incident for the entirety of the test. Nope, not until we were in the car on the way home. I was so frustrated I might have driven my car into a tree had Connor not been in the car. But the EEG came back otherwise greatly improved, the hipsarrythmia having cleared up. So this Wednesday it’s back to the neurologist for an hour EEG (yeah right, like we’ll get lucky enough for something to happen in that small window) and an appointment to discuss his progress. I’ve been a little bit crushed that Sabril wasn’t the 100 percent miracle cure it has been for many others. I truly thought it would knock them out completely within a few days, so that we’re still seeing anything has been a bit crushing. That being said, it has immensely improved the situation and allowed him to continue on his developmental path.
What we’re currently seeing: